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BACKGROUND CONTEXT: Giant cell tumor of the tendon sheath (GCTTS) is a common, benign lesion of the synovial membrane that occurs more often in large joints than in digits. Giant cell tumor of the tendon sheath rarely arises in close proximity to the axial skeleton. PURPOSE: The purpose of the study was to report a rare case of GCTTS arising from the membrane surrounding the posterior arch of the atlas (C1). STUDY DESIGN/SETTING: This is a case report. METHODS: The methods involve clinical findings and review of current literature. RESULTS: In this report, we describe a rare case of GCTTS arising from the membrane surrounding the posterior arch of C1, with no apparent continuity with the facet joint. Here we show the radiographic features, with particular emphasis on positron emission tomography-computerized tomography scans, which have not been previously reported. CONCLUSIONS: We experienced an extremely rare case of GCTTS arising from the membrane surrounding the posterior arch of the C1 vertebra. In spite of the rarity of this disease, GCTTS should be considered in the differential diagnosis of the axial skeletal lesion. Awareness of GCTTS is important because its radiographic features may simulate other neoplastic lesions in the spine.
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Tumor de Células Gigantes de Bainha Tendinosa/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Tumor de Células Gigantes de Bainha Tendinosa/etiologia , Tumor de Células Gigantes de Bainha Tendinosa/patologia , Humanos , Pessoa de Meia-Idade , CintilografiaRESUMO
STUDY DESIGN: This is a retrospective, single-institute, radiographical study. OBJECTIVE: The study aimed to determine the correlation of magnetic resonance imaging (MRI) findings observed in metastatic spinal cord compression (MSCC) with post-treatment ambulatory status. SUMMARY OF BACKGROUND DATA: Previous studies have reported various predictors of ambulatory outcome in patients with MSCC, but the relationship between the MRI features and post-treatment ambulatory function remains to be elucidated. METHODS: Fifty-six hospitalized patients with MSCC and risk of MSCC were examined using MRI before therapeutic intervention. Circumferential ratio of cord compression (CRCC), clock position of compression, cross-sectional area (CSA), and change in signal intensity of the spinal cord were recorded. Each imaging feature was analyzed statistically regarding unassisted ambulatory status at the time of hospital discharge as the endpoint. RESULTS: CRCC showed a prognostic value for post-treatment ambulatory function. More than half of CRCC predicted poor functional prognosis with statistical significance. However, the site of cord compression expressed by clock position on axial plane showed no relationship with functional prognosis. CSA of the spinal cord was enlarged in 23% of patients at the level of MSCC, which indicated that cord compression could also be formed by a relative relationship between cord swelling and surrounding mass effect. The said patients showed a better functional outcome. High intensity of the spinal cord on T2-weighted sagittal image was not useful because of lack of inter-rater reliability. CONCLUSION: CRCC on axial T2 image can guide clinicians to identify cancer patients at risk of paraplegia because of MSCC. More than half of CRCC entails urgent treatment despite preserved ambulatory function. Furthermore, some cases of MSCC accompany increased cord CSA. The measurement is also a useful guide to balance the risk and benefit of systemic steroid therapy. MRI is the key imaging modality in the risk assessment of MSCC. LEVEL OF EVIDENCE: 4.
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Imageamento por Ressonância Magnética/estatística & dados numéricos , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/cirurgia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Caminhada/estatística & dados numéricos , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Medição de RiscoRESUMO
Tumor-induced osteomalacia (TIO) is a rare acquired form of hypophosphatemia commonly associated with phosphaturic mesenchymal tumors (PMTs) located in the bone or soft tissue. Resection of the tumor can cure osteomalacia. Fibroblast growth factor 23 has been identified as a major pathophysiological factor responsible for phosphaturia. The majority of PMTs are benign, and malignant PMTs are uncommon. Even in rare cases, the malignant transformation of PMTs is extremely uncommon. The current study presents two cases in which the patients succumbed to malignant PMTs that developed in the pelvis. The first patient was a 35-year-old female with a malignant PMT occurring as a synchronous double cancer associated with papillary thyroid carcinoma. Diagnosis was difficult, as the multiple uptake on positron emission tomography with 18F-fluorodeoxyglucose presented as pseudofractures mimicking the metastases of thyroid carcinoma. The patient succumbed to rapidly progressive lung metastases. The second patient presented with a pelvic tumor that had developed over 26 years. The patient was diagnosed with a benign PMT by open biopsy and a complete resection was performed. However, two years later, the tumor recurred and lung metastases were observed. The patient ultimately succumbed to respiratory failure due to relapsing lung metastases and disseminated intravascular coagulation. These two cases demonstrate the potential lethality of malignant PMTs and the malignant transformation of benign PMTs. Therefore, TIO patients must be followed up even if diagnosed with a benign tumor. Although TIO is an extremely rare disease, the possibility of malignant PMTs must be recognized.
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Osteoid osteoma is a relatively common benign skeletal tumor. The traditional standard treatment has been surgical resection of the nidus. Recently, computed tomography (CT)-guided radiofrequency ablation (RFA) has gained favor as a more precise alternative due to potentially less bone destruction. However, CT-guided RFA is limited in treatment for osteoid osteoma involving complex anatomic structures such as cervical spine, pelvis, or scapula because of difficulty in approach and proximity to neurovascular structures. To solve this problem, we investigated RFA using a new real-time three-dimensional fluoroscopic navigation system. We report its technical procedure and use in a rare case of osteoid osteoma of the scapula.
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Neoplasias Ósseas/cirurgia , Ablação por Cateter/métodos , Osteoma Osteoide/cirurgia , Escápula/cirurgia , Cirurgia Assistida por Computador/métodos , Adulto , Neoplasias Ósseas/diagnóstico , Fluoroscopia/métodos , Humanos , Imageamento Tridimensional , Imageamento por Ressonância Magnética , Masculino , Osteoma Osteoide/diagnóstico , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
PURPOSE: We retrospectively evaluated our experience of transcatheter arterial embolization (TAE) of the sacral GCT with use of a spherical permanent embolic agent, superabsorbant polymer microsphere (SAP-MS) as an alternative treatment modality. MATERIALS AND METHODS: From 1997 to 2011, four patients with sacral GCT were treated with TAE. In all cases, SAP-MS was used as an embolic material. The effects of TAE were evaluated for improvement of patients' symptoms, radiographic change such as vascularity of tumor, size of tumor and occurrence of reossification. RESULTS: Of the four patients, three responded favorably to TAE with improvement in pain and neurologic symptoms with long-term follow up. Diminished vascularity, stabilization of tumor size and reossification were shown radiographically. One patient died because of tumor growth 26 months after the initial TAE. CONCLUSION: In sacral GCT, TAE using SAP-MS might be useful for symptom improvement, reossification of the lesion and stabilization of tumor size.
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BACKGROUND: Although intensive studies have been conducted to clarify the incidence, risks, and management methods of deep infection of tumor endoprostheses, limited data have been published in respect of the impact of such deep infection on the function of the corresponding limb. METHODS: Clinical data of 125 patients (infection group 57, control group 68) with malignant bone and soft tissue tumors around the knee enrolled with the Japanese Musculoskeletal Oncology Group were collected. We analyzed the impact of deep infection of tumor endoprostheses on the limb salvage status together with that on the function of the salvaged limb. The definition of deep infection was based on the recommendations of the Centers for Disease Control and Prevention. The functional evaluation was based on the functional classification system established by the International Society of Limb Salvage and the Musculoskeletal Tumor Society. RESULTS: Infection together with extracapsular resection was demonstrated to be a risk factor for late amputation. There were no significant differences in the functional scores for "pain," "support," "walking," or "gait" between the infection and control groups. The risk factors identified for a loss of score for "functional activities" were deep infection, age, duration of operation, and extracapsular resection. The infection group also showed a significant lower score loss in "emotion". As for the overall functional scores, the risk factors identified for lower scores were deep infection and age. The mean scores for the infection group and control group were 19.3 (64.3 %) and 21.6 (72 %), respectively. Although the difference was confirmed to be statistically significant, the actual difference was only 2.3 (10.6 % reduction). CONCLUSIONS: Infection was a major risk factor for late amputation. Limbs salvaged by management of deep infection may show loss of function; however, the impact may be limited.
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Neoplasias Ósseas/cirurgia , Articulação do Joelho , Prótese do Joelho/efeitos adversos , Infecções Relacionadas à Prótese/etiologia , Infecções Relacionadas à Prótese/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Adulto , Amputação Cirúrgica , Feminino , Humanos , Joelho , Salvamento de Membro , Masculino , Recuperação de Função Fisiológica , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Adulto JovemRESUMO
We present 2 cases of tumor-induced osteomalacia (TIO). Both patients had histories of long-term bone and muscle pain. Laboratory data revealed hypophosphatemia. Whole-body magnetic resonance (MR) imaging (WB-MRI) clearly depicted a small subcutaneous mass in the left thigh of the first patient and a right acetabular mass in the second patient. These lesions were pathologically proven to be hemangiopericytoma-phosphaturic mesenchymal tumors (PMT).
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Hipofosfatemia/etiologia , Hipofosfatemia/patologia , Imageamento por Ressonância Magnética/métodos , Mesenquimoma/complicações , Mesenquimoma/patologia , Osteomalacia/etiologia , Osteomalacia/patologia , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Pessoa de Meia-Idade , Imagem Corporal Total/métodosRESUMO
BACKGROUND: The incidence of endoprosthesis failure has been well studied, but few studies have described the clinical characteristics of deep infection in tumor prostheses. This study aimed to analyze the characteristics of deep infection in tumor endoprostheses around the knee. METHODS: We analyzed clinical data of 57 patients with deep infections involving tumor endoprostheses around the knee enrolled from the Japanese Musculoskeletal Oncology Group. Profile of clinical presentation including time between surgery and infection, initial symptoms/blood tests and microbial cultures was evaluated. In addition pre-, intra-, and postoperative clinical factors influencing clinical presentation and treatment outcomes of infections were analyzed. RESULTS: Mean interval between the initial operation and diagnosis was 13 months, and mean time required for infection control was 12 months. The most common pathogen was Staphylococcus. Infection control rates were significantly higher when prostheses were removed rather than salvaged. Ten-year prosthesis survival and limb salvage rates were 41.6% and 75.6%, respectively. Analysis of underlying clinical factors suggested that soft-tissue condition significantly influenced the duration of the infection control period and likelihood of limb salvage. CONCLUSIONS: Infection control is a prolonged process. Deep infection frequently results in amputation or prosthesis loss. Intensive analysis of clinical characteristics may aid infection control.
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Artroplastia do Joelho/efeitos adversos , Artroplastia do Joelho/instrumentação , Neoplasias Ósseas/cirurgia , Prótese do Joelho/efeitos adversos , Infecções Relacionadas à Prótese/microbiologia , Infecções Estafilocócicas/microbiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Amputação Cirúrgica , Neoplasias Ósseas/patologia , Criança , Remoção de Dispositivo , Feminino , Humanos , Controle de Infecções , Japão , Estimativa de Kaplan-Meier , Salvamento de Membro , Masculino , Pessoa de Meia-Idade , Infecções Relacionadas à Prótese/diagnóstico , Infecções Relacionadas à Prótese/terapia , Reoperação , Estudos Retrospectivos , Fatores de Risco , Infecções Estafilocócicas/diagnóstico , Infecções Estafilocócicas/terapia , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
From 1997 to 2003, 40 patients (all <40 years of age) with non-metastatic osteosarcoma of the extremities were treated with OOS-D and definitive surgery. Two cycles of doxorubicin 90 mg/m(2) plus cisplatin 120 mg/m(2) and ifosfamide 15 g/m(2) were given as neoadjuvant chemotherapy, and two cycles of doxorubicin/cisplatin and ifosfamide, and two cycles of high-dose methotrexate (10-12 g/m(2)) were given post-operatively. All patients underwent limb salvage surgeries, and 66% showed good response to neoadjuvant chemotherapy. With a median follow-up period of 117 months, 31 of the evaluable 40 patients were continuously disease-free, 7 were currently alive with no evidence of disease, and 2 died of disease. There was no local recurrence. The 5-year event-free and overall survival rates were 83 and 98%, respectively. The 10-year event-free and overall survival rates were 80 and 95%, respectively. The major form of toxicity was haematological one.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Osteossarcoma/tratamento farmacológico , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Quimioterapia Adjuvante/efeitos adversos , Quimioterapia Adjuvante/métodos , Criança , Pré-Escolar , Cisplatino/administração & dosagem , Cisplatino/efeitos adversos , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Doxorrubicina/administração & dosagem , Doxorrubicina/efeitos adversos , Extremidades/patologia , Extremidades/cirurgia , Feminino , Seguimentos , Humanos , Ifosfamida/administração & dosagem , Ifosfamida/efeitos adversos , Lactente , Recém-Nascido , Japão , Salvamento de Membro/métodos , Masculino , Metotrexato/administração & dosagem , Metotrexato/efeitos adversos , Terapia Neoadjuvante/efeitos adversos , Terapia Neoadjuvante/métodos , Osteossarcoma/patologia , Osteossarcoma/cirurgia , Taxa de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
Synovial chondromatosis of the small joints of the hand is rare. Here, we present a case of synovial chondromatosis in the metacarpophalangeal joint that did not show calcification on radiographs. Synovectomy and resection of cartilagenous loose bodies was successful and there was no recurrence after eight months.
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Artroscopia/métodos , Condromatose Sinovial/cirurgia , Articulação Metacarpofalângica , Condromatose Sinovial/diagnóstico , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-IdadeRESUMO
Liposarcomas are separated into clinicopathological entities by a characteristic morphological spectrum and distinctive genetic changes. Myxoid liposarcoma (MLS) represents one such entity with specific chromosomal translocations leading to the generation of fusion genes, the human translocation liposarcoma (TLS)-CCAAT/enhancer binding protein (C/EBP) homologous protein (CHOP) or the Ewing sarcoma (EWS)-CHOP. In the present study, four cases of liposarcoma with detection of TLS-CHOP or EWS-CHOP, whose postoperative diagnosis was other than MLS (one well-differentiated liposarcoma, two de-differentiated liposarcomas and one unclassified) were examined for medical records, imaging data and histopathology. Clinical records demonstrated that three of the four cases were considerably difficult to diagnose definitively, and histopathological re-examination pointed out areas of myxomatous change as a minor component (<10%). Their dominant components (>90%) resembled pleomorphic sarcoma, pleomorphic malignant fibrous histiocytoma and monophasic synovial sarcoma. The current cases may represent an extreme variant of the morphological spectrum within MLS. In cases of difficulty in making definitive diagnosis of soft tissue sarcoma by standard histopathological examination and identification of myxoid stroma even as a minor component, analyzing TLS-CHOP and EWS-CHOP fusion genes may aid the diagnosis of unusual MLS.
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BACKGROUND: Treatment of soft-tissue sarcomas involving the inguinal region remains challenging because of difficulties in achieving wide surgical margins due to anatomical features. The study aimed to analyze the oncologic and functional outcomes of wide resection with vascular reconstruction for inguinal soft-tissue sarcomas. METHODS: Three men and seven women were treated for inguinal soft-tissue sarcomas by wide surgical resection with vascular reconstruction. RESULTS: Arteries and veins were replaced in nine patients, and artery replacement alone was carried out in one patient. Femoral nerve resections were performed in six patients. One patient and five patients developed local recurrence and distant metastases, respectively. Limb salvage was achieved in 9 of 10 patients (90%). Six patients and one patient developed vascular (arterial graft occlusion [n = 1], lymphedema [n = 5]) and nonvascular (hematoma [n = 1]) complications, respectively. Five-year arterial primary patency was 77%. Five-year disease-free and overall survival rates were 45% and 77%, respectively. Functional outcome scores at latest follow-up averaged 87.5% for Musculoskeletal Tumor Society 1993. CONCLUSIONS: En-bloc resection of major critical structures along with tumor and vascular reconstructions using synthetic grafts is a feasible option in limb salvage surgery for inguinal soft-tissue sarcomas.
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Implante de Prótese Vascular , Extremidade Inferior/irrigação sanguínea , Procedimentos de Cirurgia Plástica , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Adolescente , Adulto , Idoso , Amputação Cirúrgica , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/mortalidade , Criança , Intervalo Livre de Doença , Feminino , Artéria Femoral/patologia , Artéria Femoral/cirurgia , Veia Femoral/patologia , Veia Femoral/cirurgia , Humanos , Artéria Ilíaca/patologia , Artéria Ilíaca/cirurgia , Veia Ilíaca/patologia , Veia Ilíaca/cirurgia , Japão , Estimativa de Kaplan-Meier , Salvamento de Membro , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Procedimentos de Cirurgia Plástica/efeitos adversos , Procedimentos de Cirurgia Plástica/mortalidade , Reoperação , Sarcoma/mortalidade , Sarcoma/secundário , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Fatores de Tempo , Resultado do Tratamento , Grau de Desobstrução Vascular , Adulto JovemRESUMO
The continuous release of bone-stored growth factors after bone resorption promotes the colonization of circulating cancer cells. However, the precise role of each of the various growth factors remains unclear. In this study, we investigated the role of bone-derived insulin-like growth factor (IGF) in the development of bone metastases in an animal model of breast cancer. We found that local stimulation of calvarial bone resorption before cell inoculation stimulated subsequent bone metastases to that site in vivo, although inhibition of bone resorption inhibited bone metastases. Anchorage-independent growth of cancer cells was stimulated by the culture supernatants from resorbed bones, which contained elevated levels of IGF-I. This stimulation was blocked by IGF type I receptor (IGF-IR) neutralizing antibody, but not antibody targeting other bone-stored growth factors including TGF-ß, fibroblast growth factors, and platelet-derived growth factors. Although recombinant human IGF-I caused IGF-IR tyrosine autophosphorylation, followed by activation of Akt and NF-κB in cancer cells, dominant-negative inhibition of IGF-IR, Akt, or NF-κB significantly reduced bone metastases with increased apoptosis and decreased mitosis in metastatic cells. Together, our findings suggest that bone-derived IGF-I bridges the crosstalk between bone and metastasized cancer cells via activation of the IGF-IR/Akt/NF-κB pathway. Disruption of this pathway therefore may represent a promising therapeutic intervention for bone metastasis.
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Neoplasias Ósseas/secundário , Neoplasias da Mama/patologia , Comunicação Celular/fisiologia , Somatomedinas/metabolismo , Animais , Neoplasias Ósseas/genética , Neoplasias Ósseas/metabolismo , Neoplasias Ósseas/patologia , Reabsorção Óssea/patologia , Neoplasias da Mama/genética , Neoplasias da Mama/metabolismo , Linhagem Celular Tumoral , Feminino , Humanos , Fator de Crescimento Insulin-Like I/metabolismo , Fator de Crescimento Insulin-Like I/farmacologia , Camundongos , Camundongos Endogâmicos BALB C , NF-kappa B/metabolismo , Proteína Oncogênica v-akt/metabolismo , Receptor IGF Tipo 1/biossíntese , Receptor IGF Tipo 1/genética , Proteínas Recombinantes/farmacologia , Transdução de Sinais/efeitos dos fármacos , Somatomedinas/farmacologia , TransfecçãoRESUMO
NF-κB is involved in the metastasis of malignant cells. We have shown that NF-κB activation is involved in the pulmonary metastasis of LM8 cells, a highly metastatic subclone of Dunn murine osteosarcoma cells. Recently, it was determined that a newly identified type of polyubiquitin chain, a linear polyubiquitin chain, which is specifically generated by the linear ubiquitin chain assembly complex (LUBAC), plays a critical role in NF-κB activation. Here, we have evaluated the roles of LUBAC-mediated NF-κB activation in the development of lung metastasis of osteosarcoma cells. All three components of LUBAC (HOIL-1L, HOIP and SHARPIN) were highly expressed in LM8 cells compared to Dunn cells. Attenuation of LUBAC expression by stable knockdown of HOIL-1L in LM8 cells significantly suppressed NF-κB activity, invasiveness in vitro and lung metastasis. Induction of intracellular adhesion molecule-1 (ICAM-1) expression by LUBAC is involved in cell retention in the lungs after an intravenous inoculation of tumor cells. Moreover, we found that knockdown of LUBAC decreased not only the number but also the size of the metastatic nodules of LM8 cells in the lungs. These results indicate that LUBAC-mediated NF-κB activation plays crucial roles in several steps involved in metastasis, including extravasation and growth of osteosarcoma cells in the lung, and that suppression of LUBAC-mediated linear polyubiquitination activity may be a new approach to treat this life-threatening disease of young adolescents.
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Neoplasias Ósseas/patologia , Neoplasias Pulmonares/secundário , NF-kappa B/metabolismo , Osteossarcoma/secundário , Animais , Proteínas de Transporte/genética , Proteínas de Transporte/metabolismo , Linhagem Celular Tumoral , Movimento Celular , Feminino , Técnicas de Silenciamento de Genes , Genes Reporter , Peptídeos e Proteínas de Sinalização Intracelular , Luciferases/biossíntese , Luciferases/genética , Neoplasias Pulmonares/metabolismo , Metaloproteinase 2 da Matriz/metabolismo , Metaloproteinase 9 da Matriz/metabolismo , Camundongos , Camundongos Endogâmicos C3H , Invasividade Neoplásica , Transplante de Neoplasias , Osteossarcoma/metabolismo , Transcrição Gênica , Ubiquitina-Proteína Ligases/genética , Ubiquitina-Proteína Ligases/metabolismoRESUMO
Intraosseous hemangioma in a rib is extremely rare, and most of the few reported cases are of the cavernous subtype. First we describe a capillary hemangioma arising from a rib in a 64-year-old woman that developed into a large, one-sided pleural effusion during the course of a 3-year follow-up. In addition to the life-threatening condition, the tumor demonstrated malignant imaging features such as a sunburst-like appearance or cortical disruption on plain roentgenogram and computed tomography. This case report adds to the literature on a serious complication and also discusses the diagnosis and management of this rare disease.
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Neoplasias Ósseas/diagnóstico , Hemangioma Capilar/diagnóstico , Costelas , Neoplasias Ósseas/complicações , Feminino , Hemangioma Capilar/complicações , Humanos , Pessoa de Meia-Idade , Derrame Pleural Maligno/etiologiaRESUMO
OBJECTIVE: Cardiac metastasis is a highly life-threatening condition because it leads to cardiac failure. However, it is difficult to diagnose because its precise clinical features are unknown. Here, we report 11 cases of cardiac metastasis from soft-tissue sarcoma, and discuss its diagnosis and treatment. METHODS: Of 641 patients with soft-tissue sarcoma treated in our institute between 1996 and 2009, we retrospectively reviewed the medical records of 11 patients whose cardiac metastases were diagnosed while they were alive. RESULTS: The most common primary tumor was leiomyosarcoma (n= 5), followed by clear cell sarcoma (n= 2). In all cases, metastases to other organs, including lungs (n= 10), soft tissues (n= 5) and bones (n= 4) were found along with cardiac metastases. Cardiac metastasis was diagnosed by echocardiography in six cases and by computed tomography in four cases. In four patients, cardiac metastasis was not detected by chest computed tomography as follow-up to lung metastases and echocardiography was required to make the diagnosis. Although five patients complained of exertional dyspnea, four were asymptomatic. Seven cases were treated with radiotherapy. No patient had surgery for their cardiac metastasis. The median survival of patients who received radiation therapy was 10.5 months; that of those who did not was 3.5 months. CONCLUSIONS: Cardiac metastasis is often asymptomatic. Echocardiography is better than computed tomography for diagnosing cardiac metastasis, and should be considered in all patients presenting with soft-tissue metastases. Owing to the highly life-threatening nature of cardiac metastases and the possibility of soft-tissue dissemination, treatment with radiation therapy is recommended immediately on diagnosis.
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Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/secundário , Sarcoma/diagnóstico , Sarcoma/secundário , Adulto , Idoso , Tamponamento Cardíaco/etiologia , Dispneia/etiologia , Ecocardiografia , Evolução Fatal , Fadiga/etiologia , Feminino , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/radioterapia , Humanos , Leiomiossarcoma/diagnóstico , Leiomiossarcoma/secundário , Neoplasias Pulmonares/secundário , Masculino , Prontuários Médicos , Pessoa de Meia-Idade , Derrame Pericárdico/etiologia , Neoplasias Peritoneais/patologia , Dosagem Radioterapêutica , Estudos Retrospectivos , Sarcoma/diagnóstico por imagem , Sarcoma/radioterapia , Sarcoma de Células Claras/diagnóstico , Sarcoma de Células Claras/secundárioRESUMO
The proximal tibia is a common site for primary bone tumors. Proximal tibial tumors may invade the adjacent soft-tissue by destroying the cortex and may further invade neurovascular bundles. We treated a patient with primary bone tumor of the proximal tibia with neurovascular invasion by extracorporeally irradiated autograft-prosthetic composite arthroplasty with vascular reconstruction. In cases of concomitant allograft arthroplasty and vascular reconstruction, we recommend that vascular reconstruction be performed before arthroplasty to minimize ischemia time. Good oncological and functional outcomes were achieved 75 months after surgery. Therefore, this reconstruction technique can be considered as a good treatment option.
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Artroplastia de Substituição/instrumentação , Implante de Prótese Vascular/instrumentação , Prótese Vascular , Neoplasias Ósseas/cirurgia , Transplante Ósseo , Veia Safena/transplante , Tíbia/cirurgia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/patologia , Quimioterapia Adjuvante , Feminino , Humanos , Salvamento de Membro , Imageamento por Ressonância Magnética , Terapia Neoadjuvante , Invasividade Neoplásica , Politetrafluoretileno , Desenho de Prótese , Tíbia/irrigação sanguínea , Tíbia/patologia , Tíbia/efeitos da radiação , Tomografia Computadorizada por Raios X , Transplante Autólogo , Resultado do Tratamento , Adulto JovemRESUMO
Nuclear factor (NF)-κB has been shown to be associated with cancer resistance to radiotherapy (RT), and is constitutively active in the murine osteosarcoma cell line, LM8. Parthenolide has been reported to show antitumor activity through inhibition of the NF-κB pathway. In this study, we investigated the radio-sensitizing activity of parthenolide. We established Luc-LM8, a stable transfectant reporter construct of NF-κB transcriptional activity into LM8. Luc-LM8 maintained the malignancy observed with LM8. In vitro, Luc-LM8 cells were cultured with or without parthenolide treatment, irradiated, and subjected to cell viability and apoptosis assays. In vivo, to investigate whether parthenolide enhances radio-sensitivity of tumors, a tumor growth assay was conducted. Parthenolide enhanced the growth inhibitory effect of RT and induced the apoptosis of Luc-LM8 cells with RT in vitro. The in vivo tumor growth was significantly suppressed in the mice treated with parthenolide and RT. The present study suggests that parthenolide sensitizes Luc-LM8 cells to irradiation. Thus, parthenolide is a potential candidate for use as a potent radio-sensitizing drug for use in cancer RT.
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Synovial sarcoma (SS) is a malignant soft tissue tumor characterized by its unique t(X;18)(p11;q11) chromosomal translocation leading to the formation of the SS18-SSX fusion gene. The resulting fusion protein product is considered to play as an aberrant transcription factor and transform target cells by perturbing their gene expression program. However, the cellular origin of SS is highly debated. We herein established two novel human SS cell lines, named Yamato-SS and Aska-SS, and investigated their biological properties. We found the self-renewal ability of these cells to generate sarcospheres, to form tumors in serial xenotransplantation and reconstitute the tumor phenotypes without fractionation by any surface markers. Both SS cells as well as clinical tissue specimens from 15 patients expressed the marker genes-associated stem cell identity, Oct3/4, Nanog, and Sox2. We also found that both SS cells displayed limited differentiation potentials for mesenchymal lineages into osteocytes and chondrocytes albeit with the expression of early mesenchymal and hematopoietic lineage genes. Upon SS18-SSX silencing with sequence-specific siRNAs, these SS cells exhibited morphological transition from spherical growth in suspension to adherent growth in monolayer, additional expression of later mesenchymal and hematopoietic lineage genes, and broader differentiation potentials into osteocytes, chondrocytes, adipocytes, and macrophages in appropriate differentiation cocktails. Collectively, these data suggest that a human multipotent mesenchymal stem cell can serve as a cell of origin for SS and SS is a stem cell malignancy resulting from dysregulation of self-renewal and differentiation capacities driven by SS18-SSX fusion protein.
Assuntos
Células-Tronco Neoplásicas/metabolismo , Sarcoma Sinovial/metabolismo , Animais , Técnicas de Cultura de Células , Diferenciação Celular , Linhagem da Célula , Regulação Neoplásica da Expressão Gênica , Humanos , Camundongos , Camundongos Nus , Células-Tronco Neoplásicas/citologia , Proteínas de Fusão Oncogênica/genética , Proteínas de Fusão Oncogênica/metabolismo , RNA Interferente Pequeno/genética , Sarcoma Sinovial/genética , Sarcoma Sinovial/patologia , Células Tumorais Cultivadas , Ensaios Antitumorais Modelo de XenoenxertoRESUMO
Synovial sarcoma (SS) is an aggressive soft-tissue malignancy characterized by a unique t(X;18) translocation resulting in expression of SS18-SSX fusion protein. In order to investigate the biological function of this fusion protein and to develop a novel therapeutic option, we examined downregulation of SS18-SSX1 expression by small interfering RNA targeting SS18-SSX1 in three human SS cell lines. Microarray analysis comparing SS18-SSX1-silenced cells with control cells in three SS cell lines showed that SS18-SSX1 mainly affected the focal adhesion pathway. In accord with the array data, silencing of SS18-SSX1 enhances adhesion to the extracellular matrix through the induction of expression of myosin light-chain kinase. Furthermore, the silencing of SS18-SSX1 inhibits anchorage-independent growth in vitro and systemic delivery of siRNA against SS18-SSX1 using a nanoparticle system inhibited tumor growth in a nude mouse xenograft model. Our results demonstrate that siRNA targeting of SS18-SSX1 has therapeutic potential for the treatment of SS.